Case: Calcified Finger Pulps
Reported by Cihangir Tetik, MD, Marmara University Hospital, Department of Orthopedics
15 year old F. She has deposits which look like calcification at her finger pulps; otherwise normal. She has 3 years history. Laboratory findings are normal. She has been referred us by Dermatology.
Does anyone have experience with this pathology? Thank you for your suggestions about the diagnosis and the treatment
FROM: Reverberi Sandro
I think it's calcinosis of fingers; it can be a part of CREST syndrome or other collagenopathies. You should find the other signs, if present (often they are not all present). You should do capillaroscopy, LE phenomenon (it's so in English language too?), auto-antibodies, etc.
Divisione di Ortopedia e Traumatologia
Azienda Ospedaliera ASMN, Reggio Emilia
FROM: Freih Odeh Abu Hassan, MD Orth, FRCS (Eng), FRCS (Tr&Orth)
Your case is most likely to be tumoral calcinosis. I saw the same picture in a patient on hemodialysis for chronic renal failure.
Freih Odeh Abu Hassan, MD Orth, FRCS (Eng), FRCS (Tr&Orth)
Assistant Professor of Orthopedics and Pediatrics Orthopedics Surgery
University of Jordan, Amman, Jordan
FROM: S. Luboshitz
Finger tip calcinosis could be manifested intracutaneous or subcutaneous among other components of scleroderma. The finger tip is tender and makes it impossible to pick up objects; sometimes the skin breaks and the deposits extrude as shown in your photos. You could find it in 15% of patients with diffuse type of scleroderma and in 45% in the limited cutaneous variety.
Surgical evacuation of the deposits is recommended while leaving the compromised skin for secondary healing rather than using mutilating needles and sutures. Recurrence is not infrequent
FROM: Myles Clough
Editor's Comments: I have no personal experience with this condition but base these comments on the bibliography which was assembled for this case. It would appear that a large number of cases of digital calcification are related to chronic renal failure and hyperparathyroidism; so it would be prudent to rule this out. It may already have been done as Dr Tetik stated that the patient's biochemistry was normal. The possible association with scleroderma or with the CREST syndrome (Calcinosis, Raynaulds, Esophagitis, Sclerodactyly, Telangectasis) should also be examined but the literature suggests that there are a number of idiopathic cases in which the only abnormality is the calcinosis.
Note that these comments are based on the diagnosis of the condition as "tumorous calcinosis." Are there any other suggestions? The Xray papers suggested that one could see "chicken wire" pattern of radiolucencies in the calcific area corresponding to fibrous septae that are found in the specimen. I thought this pattern was present in the Xray. The other pathognomonic Xray sign mentioned was fluid levels in the deposits. I assume these are only seen in larger deposits.
Orthopaedic Surgeon, Kamloops, British Columbia, Canada
Dr. Hassan sent the following set of abstracts:
Kauschke T, Zilch H Klinikum Grosshadern. Surgical treatment of Thibierge-Weissenbach syndrome and other calcinoses of the hand. Case report and review of the literature. Handchir Mikrochir Plast Chir 1999 Jan;31(1):57-60. German.
Soft-tissue calcifications in the hands may often be a sign of a systemical disease. The calcification then appears as a secondary manifestation of this disease. In that case, operative treatment ought to be a part of the overall treatment of the primary disease. When operating on fingers, the surgeon must pay attention not to compromise blood supply.
Strumia R, Lombardi AR, Bedani PL. Benign nodular calcification and calciphylaxis in a haemodialysed patient. J Eur Acad Dermatol Venereol 1998 Jul;11(1):69-71
Several types of soft tissue calcification can be detected from radiographic evaluation of patients with end-stage renal failure. The factors that predispose to such calcification include an increase in CaxP product in serum, the degree of secondary hyperparathyroidism, the level of blood magnesium, the degree of alkalosis, and the presence of local tissue injury. Three major varieties include calcification of medium-sized arteries, periarticular or tumoral calcification and visceral calcification. Calciphylaxis is a phenomenon consisting of acute ischemic necrosis in presence of calcification of dermohypodermic arterioles. It occurs mostly in chronic renal failure patients with secondary or tertiary hyperparathyroidism with a persistently elevated calcium-phosphorus product. There are few options in treating calciphylaxis and the outcome is generally poor. The authors report the case of a haemodialised patient with benign nodular calcification and calciphylaxis. The coexistence of both entities in the same patient has never been described.
Eckardt J, Towfigh H. Hypercalcinosis of the hands in scleroderma. 2 case reports. Handchir Mikrochir Plast Chir 1994 Nov;26(6):330-4. German.
Hypercalcinosis of the hand in association with scleroderma represents a rare and benign soft-tissue calcification. Reported are two cases of symmetric affection of soft-tissue layers of both hands in association with scleroderma. Superinfection and exulcerations necessitated surgical treatment. Diagnosis, therapy, and progress are described.
Itoga H, Jones JM. Soft tissue calcification in children with terminal transverse defects of the upper limb--is it tumoral calcinosis? J Hand Surg (Br) 1994 Oct;19(5):642-6.
We report five children with transverse defects of the upper limb who developed calcified deposits at the distal end of the limb. The lesions were excised from two children and had the typical histological appearance of tumoral calcinosis but on clinical grounds it is unlikely that this is the same condition as that previously described as tumoral calcinosis.
Asuncion GF, Tzarnas CD. Uremic tumoral calcinosis: acute hand presentations mimicking infection. J Hand Surg (Am) 1994 Sep;19(5):809-12.
Tumoral calcinosis is an uncommon condition of the hand characterized by deposition of calcium salts in the soft tissues of the extremities. The condition may be hereditary or acquired. Acquired tumoral calcinosis, also called tumoral calcification, is a rare manifestation of renal osteodystrophy due to derangement in divalent ion metabolism. Two chronic dialysis patients with tumoral calcification of the hand are presented. These cases are unusual in their rapid onset of presentation, mimicking acute infection. Prompt recognition of the condition may allow early nonsurgical intervention to preserve function.
Sperschneider H, Gunther K, Marzoll I, Kirchner E, Stein G. Calcium carbonate (CaCO3): an efficient and safe phosphate binder in haemodialysis patients? A 3-year study. Nephrol Dial Transplant 1993;8(6):530-4.
In an uncontrolled study, 22 dialysis patients (46 +/- 14 years, duration of dialysis 20 +/- 11 months) were treated with CaCO3 over a period of up to 3 years to lower their serum phosphate. The use of 4.5-9 g CaCO3 daily over a period of 9 months led to a reduction of mean serum phosphate from 2.51 to 1.51 mmol/l in 77% of patients, with a simultaneous increase in mean calcium concentration from 2.23 to 2.47 mmol/l, and an improved control of secondary hyperparathyroidism by reduction in mPTH from 1552 to 1032 pg/ml and in APH activity from 6.25 to 4.55 mumol/s/l. In long-term CaCO3 treatment of up to 3 years, however, a constant effective phosphate reduction could not be achieved. There was a progression (77%) of pre-existing microcalcification and a new appearance (42%) of microcalcification in vessels and soft-tissue areas of the hand. The percentage of patients with soft-tissue calcification increased from 43 to 67% during a treatment period of 3 years. We conclude that CaCO3 alone is not suitable on a long-term basis for phosphate reduction in dialysis patients.
Comment in: Nephrol Dial Transplant 1994;9(3):335-6.
Buschmann WR, Myers W, Sager G. Tumoral calcinosis. Case presentation and review. Orthop Rev 1989 Apr;18(4):440-2.
Tumoral calcinosis is a disease of unknown etiology with a greater prevalence among blacks, in which subcutaneous calcifications are found around the extensor surface of the hip, shoulder, hand and occasionally the knee. They must be differentiated from other more common causes of calcification.
Haher TR, Devlin VJ, Haher JN, Freeman B, Smith AG. A case report of calcinosis universalis. J Hand Surg (Am) 1984 Mar;9(2):243-5.
Calcium deposits in soft tissues without previous trauma may represent calcinosis universalis, a condition without systemic manifestations and with laboratory values of blood and urine that are consistently normal. Radiographs reveal areas of calcification with homogenous density, and microscopic examination of these deposits shows foreign body giant cell reaction without capsule formation. In this case of calcinosis universalis, wide excision and lavage of the calcium deposits were temporarily beneficial, but the deposits recurred in this patient.
Fischer E. Soft tissue calcifications of distal phalangeal tuberosities of the fingers. ROFO Fortschr Geb Rontgenstr Nuklearmed 1983 Aug;139(2):150-7. German.
Soft tissue calcification at the margin of the tuberosity of the distal phalanges of the fingers occurs in 7% of normal adults, varying between 0 and 14.5%, depending on age and sex. This type of calcification is least common in the little finger. The fingers on the right are more frequently affected than those on the left. Presumably this calcification results from mechanical injury ot the collagen fibres close to their insertion into the margin of the tuberosity.
Quarles LD, Murphy G, Econs MJ, Martinez S, Lobaugh B, Lyles KW. Uremic tumoral calcinosis: preliminary observations suggesting an association with aberrant vitamin D homeostasis. Am J Kidney Dis 1991 Dec;18(6):706-10.
Periarticular tumoral calcification is a unique form of soft tissue calcification that occurs infrequently in patients with end-stage renal disease. The mechanism underlying such massive periarticular calcifications is unknown. The radiographic similarity between uremic tumoral calcifications and those found in hereditary tumoral calcinosis, a disorder of calcitriol and phosphorus homeostasis, caused us to examine whether abnormalities in vitamin D metabolism were associated with uremic calcinosis as well. We examined two uremic subjects with massive periarticular tumoral calcifications and found that they had inappropriately high serum calcitriol levels for the degree of renal function, hyperparathyroidism, and hyperphosphatemia. The source of calcitriol could not be identified in one subject, but likely was derived from granulomatous tissue in the other. In the subject with marrow granulomas, we found that calcitonin administration further stimulated calcitriol production. Although epidemiological studies are needed to confirm this preliminary association between calcitriol and uremic tumoral calcinosis, our observations suggest that normal serum calcitriol levels in association with hyperphosphatemia may be a contributing factor in the development of this rare disorder.
Harwood CA, Cook MG, Mortimer PS. Tumoral calcinosis: an unusual cause of cutaneous calcification. Clin Exp Dermatol 1996 Mar;21(2):163-6.
Tumoral calcinosis is an uncommon ectopic calcification syndrome characterized clinically by the presence of irregular, painless, periarticular soft tissue calcifying masses, and pathologically by fibrous-walled cystic spaces containing structureless calcific debris and associated with a variable inflammatory reaction. The pathogenesis remains obscure but the condition probably represents a disordered tissue reparative process. Of the previous literature reports, almost all have been in patients of African origin. We report a case in a white English woman.
Malik M, Acharya S. Tumoral calcinosis of the fingers. A report of two cases. Int Orthop 1993 Nov;17(5):279-81.
Tumoral calcinosis is an uncommon condition which usually appears in the region of large joints. It is rare in the hand. We present two cases affecting the fingers. No causative abnormality was found.
Drueke TB. A clinical approach to the uraemic patient with extraskeletal calcifications. Nephrol Dial Transplant 1996;11 Suppl 3:37-42.
Soft tissue calcifications are a frequent complication in patients with chronic renal failure. In most instances they remain clinically silent. However, in a minority of patients they are responsible for complications and may even become life-threatening. Various locations and types of calcium deposits have been characterized. Numerous underlying factors are thought to favour their formation, in particular increased calcium x phosphate product and advanced age. In most cases, local factors probably are involved as well. Tumoral calcinosis is a rarely observed form of extraskeletal calcification which is often invalidating. Since treatment is generally difficult, prevention should be the preferred goal.
Eisenberg B, Tzamaloukas AH, Hartshorne MF, Listrom MB, Arrington ER, Sherrard DJ. Periarticular tumoral calcinosis and hypercalcemia in a hemodialysis patient without hyperparathyroidism: a case report. J Nucl Med 1990 Jun;31(6):1099-103.
We present a case of a 58-yr-old male to illustrate the scintigraphic, roentgenographic, clinical, and pathologic features of periarticular tumoral calcinosis that occurred in a hemodialysis patient. Soft-tissue calcifications developed 3 yr after onset of hemodialysis, became progressively larger during the ensuing five years, and culminated in voluntary withdrawal from dialysis because of the extreme discomfort and lack of mobility that resulted from the calcinosis. Histologically, an aplastic disorder was present with very low bone formation. On bone scintigraphy, intense calcium uptake in soft tissues implied that it was metabolically active. We hypothesize that this high metabolic activity contributed to the persistent hypercalcemia observed during the patient's last year of life.
Schenkier SL, Gertner E. Massive soft tissue calcification causing complete loss of extensor tendon function in renal failure. J Rheumatol 1992 Oct;19(10):1640-2.
Extraskeletal soft tissue calcifications occur commonly in patients with uremia receiving dialysis. Rarely, a large tumoral calcinosis-like mass may develop. A patient receiving chronic ambulatory peritoneal dialysis for only 7 months developed a tumoral calcinosis-like mass that encased the extensor tendons of his wrist with loss of extensor tendon function, initially suggesting extensor tendon rupture. Surgical debridement restored tendon function. Tumoral calcinosis-like lesions are uncommon, but may cause limitation of joint movement, pain or ulceration through the skin. Measures aimed at controlling factors contributing to soft tissue calcification should be undertaken in any event whether surgery is required or not.
Ohashi K, Yamada T, Ishikawa T, Yamaguchi S, Nakajima H, Takagi M. Idiopathic tumoral calcinosis involving the cervical spine. Skeletal Radiol 1996 May;25(4):388-90.
We report a case of a 12-year-old girl with idiopathic tumoral calcinosis of the neck. There are calcium deposits in the paraspinal soft tissue with bony involvement in the cervical spine. CT and MR images are presented along with
clinical and pathological features. Bony involvement in this disease has not been recognized before.
Walker GS, Davison AM, Peacock M, McLachlan MS. Tumoral calcinosis: a manifestation of extreme metastatic calcification occurring with 1, alpha-hydroxycholecalciferol therapy. Postgrad Med J 1977 Sep;53(623):570-3.
Two cases are reviewed, both of which developed tumoral calcinosis whilst receiving 1, alpha-hydroxy-cholecalciferol therapy. Tumoral calcinosis is an extreme form of peri-articular calcification, and its occurrence in patients
with chronic renal failure is unusual. These peri-articular masses developed around the shoulders in both patients, and the action of 1,alpha-hydroxycholecalciferol as a possible factor promoting this form of metastatic calcification is discussed.
Calloway DM, Saldana MJ. Combined modality treatment for tumoral calcinosis. Orthop Rev 1993 Mar;22(3):365-9.
Tumoral calcinosis is a rare syndrome marked by periarticular and intramuscular calcifications. We present the case of a 13-year-old black girl who has received treatment since age 2 for tumoral calcinosis with bilateral shoulder
Milliner DS, Zinsmeister AR, Lieberman E, Landing B. Soft tissue calcification in pediatric patients with end-stage renal disease. Kidney Int 1990 Nov;38(5):931-6.
Soft tissue calcification is a recognized complication of uremia in adult patients and has been implicated as a cause of ischemic necrosis, cardiac arrhythmias, and respiratory failure. However, soft tissue calcification has been regarded as rare in pediatric renal patients. Following a sudden death due to pulmonary calcinosis in an adolescent after renal transplantation, we retrospectively reviewed clinical, biochemical and autopsy data of 120 patients with uremia, on dialysis, or following renal transplantation cared for at Childrens Hospital of Los Angeles from 1960 to 1983. Soft tissue calcification was found in 72 of 120 patients (60 percent). Forty-three patients (36 percent) had systemic calcinosis (Group A): the most frequent sites of mineral deposition were blood vessels, lung, kidney, myocardium, coronary artery, central nervous system, and gastric mucosa. Vascular calcification was uniformly accompanied by deposits in other organs. Twenty-nine patients had small amounts of focal calcification (Group B) and 48 patients had no soft tissue calcification (Group C). By multiple logistic regression analysis, the use of vitamin D or its analogues, the form of vitamin D medication prescribed, the peak calcium x phosphorus product, the age at onset of renal failure, and male sex were jointly associated with calcinosis (Group A). Vitamin D therapy showed the strongest independent association with calcinosis and the probability of calcinosis was greater in patients receiving calcitriol when compared with dihydrotachysterol and vitamin D2 or D3. The duration of renal failure, peak serum calcium, serum calcium at death, serum phosphorus at death, and primary renal diagnosis, were not statistically associated with calcinosis.(ABSTRACT TRUNCATED AT 250 WORDS)
Cofan F, Garcia S, Combalia A, Campistol JM, Oppenheimer F, Ramon R. Uremic tumoral calcinosis in patients receiving longterm hemodialysis therapy. J Rheumatol 1999 Feb;26(2):379-85.
OBJECTIVE: To analyze a series of uremic tumoral calcinosis (UTC) in patients receiving longterm dialysis therapy. METHODS: Twelve patients receiving longterm hemodialysis affected by tumoral calcinosis were analyzed. Clinical,
radiological, and pathological features were evaluated and pathogenic factors were reviewed. RESULTS: The most common sites for UTC were the elbow, hip, hand, and wrist. The lesions were multiple (67%, n = 8), of large size, and symptomatic with joint mobility impairment (75%, n = 9) as well as nerve compression (33%, n = 4). High serum calcium and phosphate concentrations were detected in 50% (n = 6) and 100% of the patients, respectively. An increased calcium-phosphorus product (Ca x P) was observed in all patients, either due to overt secondary hyperparathyroidism (42%, n = 5), or secondary to iatrogenic hypercalcemia and/or severe hyperphosphoremia of multifactorial etiology (i.e., prolonged and excessive administration of calcitriol and calcium carbonate, insufficient dialysis and inadequate phosphorus chelating therapy, etc.) (58%, n= 7). Several treatment strategies were followed (surgical excision, parathyroidectomy, renal transplant) in combination with aggressive medical therapy to decrease Ca x P product, achieving complete remission in 83% of the patients. CONCLUSION: UTC lesions show clinical and pathogenic features that differ from those of idiopathic tumoral calcinosis. The most important pathogenic factor involved in UTC is an increase in Ca x P, not necessarily related to hyperparathyroidism. Combined treatment strategies allow complete remission in a high proportion of patients. A low Ca x P is necessary to prevent development of UTC.
Ammar A, Ben Romdhane K, Khattech R, Ben Othman M, Ben Ghachem M. Tumoral calcinosis: a clinical and pathological study of 8 cases reported in Tunisia. Rev Chir Orthop Reparatrice Appar Mot 1994;80(3):261-6. French.
Tumoral calcinosis is a distinct clinicopathological entity characterized by periarticular soft-tissue calcium deposits. It is a rare condition in Tunisia (8 cases during 23 years). Six patients were aged 14 years or younger. There was male predominance (SR: 7/1). Lesions were located about the hip in five patients and the elbow in three patients. Multiple localisations occurred in two patients. Histologically, all cases exhibited active phase of the disease. In our patients lesions were only surgically excised.
PMID: 7899646, UI: 95207544
Abraham Z, Rozner I, Rozenbaum M. Tumoral calcinosis: report of a case and brief review of the literature. J Dermatol 1996 Aug;23(8):545-50.
A patient with a 32-year history of tumoral calcinosis is described. The calcified lesions involved the soft tissues in the hips, shoulders, and ankles.Periodically, a chalky semifluid material extruded through several cutaneous sinuses. Laboratory tests including serum calcium concentration were normal, except for slight elevation of serum phosphorous levels. Ophthalmologic examination revealed the interesting finding of subretinal angioid streaks. The dental radiograms disclosed pathognomonic short bulbous roots and partial obliteration of pulp cavities, while the radiological evaluation of the tumoral masses revealed typical features of tumoral calcinosis.
Noyez JF, Murphree SM, Chen K. Tumoral calcinosis, a clinical report of eleven cases. Acta Orthop Belg 1993;59(3):249-54.
Clinical observations of 11 new cases of tumoral calcinosis are reported. The condition is characterized by calcified masses of varying size in the region of major joints. Surgical excision is recommended in selected cases determined by the size of the lesion, the deformity present and functional complaints. In this series surgical excision was done in 6 patients, and the diagnosis was confirmed histopathologically. After a complete excision of the tumor, no recurrences were seen in 5 cases with a mean follow-up time of 25 months. Incomplete excision led to multiple recurrences in one patient.
Haddad SN, Ghossain MA, Jebara VA, Roucos S, Kharrat K, Boustany FN. Tumor calcinosis. Review of the literature: apropos of a case. Rev Chir Orthop Reparatrice Appar Mot 1989;75(5):340-4. French.
Tumoral calcinosis is an uncommon disease of unknown etiology characterized by the presence of single or multiple lobulated, para-articular, cystic soft tissue productions. Hyperphosphatemia is the only biological abnormality which can be detected. The honeycomb roentgenographic pattern is characteristic. CT scan and ultrasound are useful in showing the extra-articular location of the mass. The treatment is surgical and the prognosis is good.
Tezelman S, Siperstein AE, Duh QY, Clark OH. Tumoral calcinosis. Controversies in the etiology and alternatives in the treatment. Arch Surg 1993 Jul;128(7):737-44; discussion 744-5.
OBJECTIVE: To examine our experience and review the literature concerning the diagnosis, origin, and treatment of tumoral calcinosis (TC). DESIGN/SETTING: Case series based on patients with TC treated in University of California-San Francisco hospitals from 1981 to 1992 and the review of the patients described in the English-language literature. PATIENTS: The study included a total of 17 patients: 10 women and seven men. MAIN OUTCOME MEASURES: Sex, age, origin, symptoms, localization, treatment, and morbidity. RESULTS: Seven men and six women, from 32 to 62 years of age, had known disorders of calcium metabolism, and four women, from 37 to 84 years of age, did not. The main causes of the calcium metabolic disorder were secondary hyperparathyroidism in 11 patients (85%) and primary hyperparathyroidism in two patients. In three patients there was a history of trauma at the involved site and in one patient the origin was unknown. Swelling and pain are the most common presenting complaints. Generalized pruritus was observed in 54% of the patients with metabolic disorders (P < .001) but not in patients without metabolic disorders. Among our patients with metabolic disorders, TC occurred most frequently at the shoulder (46%) and elbow (31%). Eleven patients with secondary hyperparathyroidism had received calcium carbonate to bind phosphate, a high level of calcium in the dialysate, and calcitriol (1,25-vitamin D) either orally, intravenously, or both, and three received epoetin alfa (Epogen). Following parathyroidectomy, the patients with hyperparathyroidism improved symptomatically, although calcifications did not change in size. One patient had the calcifications resected and did well, whereas another was treated by subtotal resection and had a recurrence 3 years later. All four of our patients without a metabolic disorder had complete resection of TC with no recurrence. CONCLUSION: We believe TC is becoming more common in uremic patients with secondary hyperparathyroidism because of recent changes in the medical treatment of these patients. The
increased use of calcium carbonate to bind phosphate as well as calcitriol and calcium to suppress parathyroid function and possibly epoetin alfa are causing more patients to develop TC.
Mills CM, Knight AG. Cutaneous calcinosis--an unusual complication of intravenous phosphate administration. Clin Exp Dermatol 1993 Jul;18(4):370-2.
The case of an 80-year-old woman who developed extensive cutaneous calcification following intravenous phosphate administration is presented. Also the circumstances under which cutaneous calcification may occur are discussed.
Roe SM, Graham LD, Brock WB, Barker DE. Calciphylaxis: early recognition and management. Am Surg 1994 Feb;60(2):81-6.
Calciphylaxis, a syndrome of disseminated calcification found in chronic renal failure patients with secondary hyperparathyroidism, results in soft tissue calcification and vascular medial calcinosis leading to subsequent ischemic tissue necrosis. It is a rarely occurring condition in which patients present with painful, violaceous, mottled lesions of the extremities and/or trunk that progress to skin and subcutaneous tissue necrosis, non-healing ulcers, and gangrene. We reviewed the clinical course of seven patients (aged 24-69) with calciphylaxis treated at our institution over a 4-year period (October 1988-June 1992). All seven patients underwent parathyroidectomy, with a mean time of 8 weeks (range 3-20 weeks) between the onset of calciphylactic symptoms and parathyroidectomy. Four patients died, three secondary to wound-related sepsis. Of the three survivors, two healed soft tissue lesions primarily. The other required extremity amputation and wound excision before healing. Neither anatomical location of the soft tissue lesions nor post-parathyroidectomy serum calcium and phosphorus levels had any bearing on wound healing or mortality. Lesion severity at the time of parathyroidectomy appeared to best correlate with clinical course. Although treatment with phosphate-binding antacids, total or subtotal parathyroidectomy, and avoidance of challengers such as Vitamin D or local tissue trauma remain the mainstays of therapy, the uniform cure for calciphylaxis remains elusive. Prognosis for patients with calciphylaxis is dismal, even following late surgical intervention. Earlier recognition of the signs and symptoms of calciphylaxis should lead to timely parathyroidectomy in the hopes of ameliorating the symptoms and preventing or retarding its progressive sequelae.
Wu T, Chen SS. Differential diagnosis between fibrodysplasia ossificans progressiva and childhood dermatomyositis with calcinosis. J Formos Med Assoc 1993 Jun;92(6):569-76.
Both fibrodysplasia ossificans progressiva (FOP) and childhood dermatomyositis with calcinosis are rare diseases, and present with ossifying or calcifying processes. Six cases of FOP and one case of childhood dermatomyositis with calcinosis are studied. All six FOP patients had the typical digital anomalies and the characteristic ectopic bone formation starting from the trunk. Calcification in the case of childhood dermatomyositis occurred in the limbs. A carefully differentiated diagnosis between these two diseases is needed, because they share common clinical and radiologic features, but require different management. Delay in the diagnosis of FOP is common, although early recognition of FOP prevents a child from accidental or iatrogenic injury, which can precipitate ectopic ossification. Surgical removal of the ectopic bone or release of the contracture in three FOP patients was followed by rapid recurrence. Entrapment neuropathy and a mild myopathic pattern in two FOP patients, who underwent nerve conduction and electromyographic studies, were secondary to ectopic bone formation. One FOP patient received a computed tomography examination which showed basal ganglia calcification. No coexistence of FOP and childhood dermatomyositis with calcinosis was found.
Alfrey AC, Solomons CC. Bone pyrophosphate in uremia and its association with extraosseous calcification. J Clin Invest 1976 Mar;57(3):700-5.
The mean bone pyrophosphate was 0.360 +/- 0.15 mg/g in 8 controls and 1.22 +/- 1.39 mg/g bone in 27 uremic patients (P less than 0.0025). 13 of the 27 uremic patients had bone pyrophosphate levels greater than 2 SD above control values. The ash content of uremic bones with increased pyrophosphate levels (group II) was 56 +/- 9% as compared to 64 +/- 2% in control bones (P less than 0.01) and 60 +/- 7% in uremic bones having normal pyrophosphate levels (P less than 0.1) (group I). The magnesium content of bones in group II was 338 +/- 47 as compared to 211 +/- 13 (P less than 0.0005) in the controls and 294 +/- 73 mmol/kg ash (P less than 0.05) in group I. In group II, but not group I, there was a significant inverse correlation between duration of dialysis and percent bone ash (r = - 0.59) (P less than 0.05). A definite relationship existed between elevated bone pyrophosphate levels and soft tissue calcification. In group II the mean pulmonary calcium content was 530 +/- 459 as compared to 32 +/- 26 mmol/kg/ash in group I (P less than 0.0025). All patients with a bone pyrophosphate level greater than 1.4 mg/g bone had extensive pulmonary calcification. It is concluded that the excess bone pyrophosphate present in some uremic patients is either deposited in the apatite crystal in the transphosphorylated form or else as the magnesium salt since the pyrophosphate is resistant to pyrophosphatase and surface adsorption of pyrophosphate is not altered by the increased bone pyrophosphate levels. The excess bone pyrophosphate could disturb bone calcification mechanisms in uremic patients. The association between increased bone pyrophosphate and soft tissue calcification suggests that the disordered pyrophosphate metabolism may be important in the pathogenesis of extraosseous calcification.
Gregosiewicz A, Warda E, Stolecka D, Kandzierski G. Calcinosis tumoralis. Pharmacological alternatives to surgical treatment and its etiological justification. Chir Narzadow Ruchu Ortop Pol 1989;54(2):159-63. Polish.
A detailed case history of a 6 years old child, including biochemical and radiological examinations, has been presented. The disease started with a commonplace contusion of the patella and rapidly progressed after arthrotomy. Dihydroxy-aluminium-natrium carbonicum was applied as well as the low calcium and phosphorus diet. After 4 weeks of treatment, the joint recovered its correct contours; and after further 7 months, only vestigial calcification was observed.
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